MyPACS.net: Radiology Teaching Files > Case 947113

previously visited RECURRENCE OF A LOW-GRADE CERVICOMEDULLARY CEREBELLAR GLIOMA
Contributed by: Dr Phillip Silberberg, Children's Hospital Omaha, Radiologist, Omaha Childrens, Creighton University and UNMC, Nebraska, USA.
Patient: 17 year old female
History: Recurrence of a low-grade cerebellar tumor excised with micro dissection under operating microscope
Images:[small]larger

Fig. 1: MRI 3 July 2004.

Fig. 2: MRI 3 July 2004.

Fig. 3: 1/04-pilocytic astrocytoma (WHO Grade I)

Fig. 4: 1/04-pilocytic astrocytoma

Fig. 5: 1/04-Rosenthal fibers

Fig. 6: MRI 3 July 2004.

Fig. 7: MRI 3 July 2004.

Fig. 8: MRI 3 July 2004.

Fig. 9: 7/04 recurrent tumor

Fig. 10: 7/04-recurrent tumor

Fig. 11: Dec 4 04 Sag T1. resection site dorsal medulla and dorsal spinal cord

Fig. 12: Dec 4 04 Sag T1 post Gad. No enhancement

Fig. 13: Dec 4 04 Sag T2. Increased T2. Also syrinx or porencephalic defect

Fig. 14: Dec 4 04 Axial T2

Fig. 15: Dec 4 04 Axial T2. Syrinx or porencephalic defect

Fig. 16: Dec 4 04 Coronal T2. Syrinx or porencephalic defect

Fig. 17: 12/04 recurrent tumor

Fig. 18: 12/04 -recurrent tumor
Diagnosis: RECURRENCE OF A LOW-GRADE CERVICOMEDULLARY CEREBELLAR GLIOMA
Discussion:

 

The three types of glial cells give rise to three corresponding tumor types of the brain and spinal cord: astrocytoma, oligodendroglioma, and ependymoma.  While adult gliomas are largely supratentorial, 70-80% of pediatric gliomas are infratentorial.  Cerebellar gliomas represent approximately 30% of such tumors. 

 

The majority of tumors in this location are pilocytic astrocytomas by histology. Pilocytic astrocyomas comprise about one third of all childhood gliomas. These lesions are commonly well-circumscribed and may be cystic with a highly-vascularized mural nodule, or multilobulated.  

 

Symptoms and signs were delayed and protracted, often occurring over months to years The tumors expanded the dorsal medulla and involved the upper cervical spinal cord and can measure up to 4.4 cmin diameter. Hydrocephalus may develop.

Cervicomedullary pilocytic astrocytomas have a remarkably good prognosis in comparison to other pontine and brainstem gliomas.  They present in the midline with slow expansion and rare malignant transformation.  Rarely leptomeningeal seeding may occur via extension into the subarachnoid space.  It has been reported that this spread of tumor is often related to surgery.  Nonetheless, the 5-year survival approaches 90%. 

 

These tumors characteristically present in the midline and may cross to either side. They have both cystic and solid elements.  MRI is superior to CT imaging for investigation of gliomas both because of its multiplanar capabilities and because of its great contrast resolution. MRI also is advantageous due the lack of use of ionizing radiation.  The solid element is a mural nodule,  isodense on CT and isointense on MRI to the adjacent cyst wall.  CT imaging of the remainder of the gliomas will reveal a non-enhancing, hypodense space-occupying lesion.  With MRI,  the tumor is  hypointense on T1 and hyperintense on T2. On both CT and MRI, the nodule may enhance independent of the cyst wall.

 

Treatment includes surgery alone, surgery with  radiation, and combined surgery, chemotherapy, and radiation.   Radical surgery is indicated for low-grade astrocytic tumors. The need for adjuvant therapy most often depends on the extent of resection as well as the tumor type. Patients with disseminated JPA and nonpilocytic astrocytoma may achieve long-term progression-free survival with craniospinal irradiation. .  Survivors may show mild to moderate neurological dysfunction, more commomly if radiation was utilized. The commonest surgical complications are CSF leakage and akinetic mutism.

 

References:

Contributed by:
Jennifer Lee, Medical student M4, Creighton University, Omaha, NE.
Phillip Silberberg, MD. Omaha Children's Hospital, Creighton University and UNMC, Omaha, NE.

References:

  1. Medcyclopaedia.  Medical Diagnostics.  GE Healthcare.  http://www.medcyclopaedia.com/library/topics.  July 10, 2006. 
  2. Spinal seeding of pilocytic astrocytoma: Case report and review of the literature.   By: Genç, Mine; Gürkaynak, Murat; Zorlu, Abdullah Faruk; Akalan, Nejat; Söylemezoglu, Figen; Büyükpamukçu, Münevver. Turkish Journal of Cancer, Oct2005, Vol. 35 Issue 4, p186-188, 3p
  3. Prognostic Factors for Cerebellar Astrocytomas in Children: A Study of 102 Cases.   By: Desai, Ketan I.; Nadkarni, Trimurti D.; Muzumdar, Dattatraya P.; Goel, Atul. Pediatric Neurosurgery, Dec2001, Vol. 35 Issue 6, p311-317
  4. Surgical Outcomes of Cerebellar Tumors in Children.   By: Akay, Kamil Melih; Izci, Yusuf; Baysefer, Alper; Atabey, Cem; Kísmet, Erol; Timurkaynak, Erdener. Pediatric Neurosurgery, Sep/Oct2004, Vol. 40 Issue 5, p220-225

 


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Additional Details:

Case Number: 947113Last Updated: 08-20-2006
Anatomy: Cranium and Contents   Pathology: Neoplasm
Modality: PathologyAccess Level: Readable by all users
Keywords: recurrence of a low-grade cervicomedullary cerebellar glioma

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